Abstract 1122‐000115: Coiling of Wide‐Necked Ruptured Aneurysms: A Subset Analysis of the SMART Registry

Introduction : The purpose of this study was to assess the 1‐year clinical outcomes of wide‐necked ruptured aneurysms treated with coiling. Methods : Data on patients with a wide‐necked ruptured aneurysm were extracted from a prospective multicenter registry (SMART) that enrolled patients with intracranial aneurysm or other neurovascular abnormality who underwent coiling. A wide neck was defined as a neck width of at least 4 mm or as a dome‐to‐neck ratio (largest diameter / neck width) of less than 2. Enrollment was not limited by Hunt and Hess grade. The primary safety outcome was device‐related serious adverse events within 24 hours, and the primary effectiveness outcome was retreatment through follow‐up. Results : Of the 995 adults enrolled in the SMART registry, 144 had a wide‐necked ruptured aneurysm (Table). Average patient age was 59.3 years (SD 14.3), and 74.3% were female. Lesion locations were internal carotid artery, 31.3%; anterior communicating artery, 31.9%; middle communicating artery, 10.4%; and posterior circulation, 26.4%. Aneurysm sizes were small, 27.1%; medium, 54.2%; large, 18.1%; and giant, 0.7%. The most common aneurysm type was saccular (88.8%, 127/143). Coiling was stent assisted in 10.4% of patients and balloon assisted in 36.1% of patients. The rate of device‐related serious adverse events within 24 hours was 3.5%. The rate of retreatment through follow‐up was 20.6% (20/97). At 1 year, 82.6% (76/92) of patients had a Raymond–Roy Occlusion Classification of I or II, 32.6% (30/92) had progressive occlusion, and 46.7% (43/92) had stable occlusion. The 1‐year all‐cause mortality rate was 12.5%. At 1‐year follow‐up, 58.3% (42/72) of patients had a modified Rankin Scale score of 0 to 2. Conclusions : Treatment of wide‐necked ruptured aneurysms with coiling has acceptable occlusion and retreatment rates at 1‐year follow‐up.

Iron Deficiency in Pulmonary Arterial Hypertension: A Deep Dive into the Mechanisms

Pulmonary arterial hypertension (PAH) is a severe cardiovascular disease that is caused by the progressive occlusion of the distal pulmonary arteries, eventually leading to right heart failure and death. Almost 40% of patients with PAH are iron deficient. Although widely studied, the mechanisms linking between PAH and iron deficiency remain unclear. Here we review the mechanisms regulating iron homeostasis and the preclinical and clinical data available on iron deficiency in PAH. Then we discuss the potential implications of iron deficiency on the development and management of PAH.

The safety and outcomes of acutely ruptured intracranial aneurysms with incomplete occlusion after coiling: a case-control study

Background Incomplete aneurysmal occlusion is a common feature of immediate posttreatment angiography. The safety and outcomes of acutely ruptured intracranial aneurysms (RIAs) with incomplete occlusion after stent-assisted coiling (SAC) and no-stent coiling (NSC) have not been well clarified. Progressive occlusion of stents can promote the complete occlusion of intracranial aneurysms (IAs), but it remains to be determined if progressive occlusion in acutely RIAs with incomplete occlusion after coiling may be enhanced by protective stenting. This study aimed to evaluate the safety and outcomes of those aneurysms after SAC and NSC; And to discover whether the stents can promote progressive aneurysm occlusion in such lesions or not. Methods We reviewed 199 patients with acutely RIAs underwent endovascular coiling and developed incomplete occlusion in the past seven years. The patients’ clinical and imaging information were recorded and analyzed. Univariate and multivariate analyses were performed to determine the association of recurrence rate with potential risk factors. Results SAC group had wider aneurysms neck (3.471 mm vs 2.830 mm, P = 0.009) and smaller dome-to-neck ratio (1.536 vs 2.111, P = 0.001) than in NSC group. There was no significant difference between the two groups in total procedure-related complications rate (31.7% vs 23.5%, P = 0.195), procedure-related mortality (6.9% vs 2.0%, P = 0.170) and modified Rankin Scale (mRS) score at 6-month follow-up (P > 0.05). However, SAC group had significantly higher ischemic complications rate (21.8% vs 8.2%, P = 0.007) and complete occlusion rate (65.6% vs 48.3%, P = 0.020), and lower recurrence rate (15.6% vs 28.1%, P = 0.042) than NSC group based on 6-month follow-up angiograms. Additionally, Multivariable analysis showed NSC was an independent risk factor for aneurysm recurrence (Odds Ratio [OR]: 4.061; P = 0.018). Conclusions Acutely RIAs with incomplete occlusion after SAC is associated with higher complications rate and mortality, but has an acceptable safety profile and similar clinical outcome compared to NSC, as well as gives patients superior angiography outcome by progressive occlusion of stents.

MOYAMOYA DISEASE PRESENTING AS ACUTE ENCEPHALITIS SYNDROME

Background: Moyamoya disease (MMD) is a chronic, progressive occlusion of the circle of Willis arteries usually affects bilateral carotid arteries than unilateral that leads to the development of characteristic collateral vessels seen on imaging, particularly cerebral angiography. Once the occlusion process begins, tends to continue despite any known medical management unless treated with revascularization surgery Report of the case: A 28 year female gravida four in 34th week of gestation presented with features suggestive of acute meningoencephalitis and CSF examination suggested 75% polymorphs of 300 total leukocytes. After 7 days of optimum management, complaints of headache aggravated so patient was subjected to MRI Brain which reported as early subacute intraparenchymal haemorrhage left frontal lobe in periventricular region with mild surrounding perifocal edema and intraventricular extension. On further MR angiogram, patient was diagnosed as MMD. Patient was asymptomatic in her previous 3 pregnancies and presently underwent planned preterm high risk caesarean section after a week. However condition of patient deteriorated over 2 weeks post delivery, did not remain fit for any neurosurgical intervention and unfortunately succumbed to death. Conclusion: Antenatal patient presenting as acute meningoencephalitis with persistent headache refractory to medical therapy, should be subjected to CT/MR brain imaging for rare possibility of MMD and early neurosurgical interventions in view of its grave prognosis.

High Prevalence of Moyamoya Syndrome in Appalachia

<b><i>Introduction:</i></b> Moyamoya is a chronic cerebrovascular condition of unclear etiology characterized by progressive occlusion of 1 or both internal carotid arteries with neovascular collateral formation. With both an idiopathic form (moya­moya disease) and congenital condition-associated form (moyamoya syndrome), it can cause ischemic and hemorrhagic stroke. Recent findings in Kentucky have challenged traditional estimates of its incidence in US populations. Using the Kentucky Appalachian Stroke Registry (KApSR), our aim was to further characterize its incidence as a cause of stroke and to understand the patient population in Appalachia. <b><i>Methods:</i></b> A retrospective review of moyamoya patients was performed using the KApSR database. Data collected included demographics, county location, risk factors, comorbidities, and health-care encounters from January 1, 2012, to December 31, 2016. <b><i>Results:</i></b> Sixty-seven patients were identified; 36 (53.7%) resided in Appalachian counties. The cohort accounted for 125 of 6,305 stroke admissions, representing an incidence of 1,983 per 100,000 stroke admissions. Patients presented with ischemic strokes rather than hemorrhagic strokes (odds ratio 5.50, 95% CI: 2.74–11.04, <i>p</i> &#x3c; 0.01). Eleven patients (16.4%) exhibited autoimmune disorders. Compared to the general population with autoimmune disorder prevalence of 4.5%, the presence of autoimmunity within the cohort was significantly higher (<i>p</i> &#x3c; 0.01). Compared to non-Appalachian patients, Appalachian patients tended to present with lower frequencies of tobacco use (<i>p</i> = 0.08), diabetes mellitus (<i>p</i> = 0.13), and hypertension (<i>p</i> = 0.16). <b><i>Conclusions:</i></b> Moyamoya accounts for a substantial number of stroke admissions in Kentucky; these patients were more likely to develop an ischemic stroke rather than a hemorrhagic stroke. Autoimmune disorders were more prevalent in moyamoya patients than in the general population. The reduced frequency of traditional stroke risk factors within the Appalachian group suggests an etiology distinct to the population.

Moyamoya Vasculopathy Presenting with Transient Neurological Deficit

This chapter addresses the management of patients with moyamoya vasculopathy presenting with transient neurological deficit. Progressive occlusion of the major intracranial arteries at the intradural carotid bifurcation with collateral formation is the hallmark of moyamoya vasculopathy. This rare disorder can cause both hemorrhagic and ischemic stroke, and it is often encountered in younger patients. The radiographic stage of the disease can help estimate both the risk of stroke and the need for treatment, although decision-making must be considered in the context of the degree of clinical symptomatology. Surgical management includes revascularization with extracranial–intracranial bypass, and careful patient selection and surgical technique are required to improve patient outcomes.

Bilateral Cerebrovascular Stroke as an Initial Presenting Symptom of Moyamoya Disease

Moyamoya disease is a rare condition affecting the circle of Willis and its branching arteries. While the pathogenesis is unclear, it causes progressive occlusion of multiple cerebral vessels leading to severe strokes. We report a case of a 47-year-old Hispanic woman with HTN presented with altered mental status and bilateral upper and lower extremity weakness with dystonic-like upper extremity movement. Serial brain CTs and angiography were performed which showed massive frontal and parietal cerebral infarcts with radiological evidence of moyamoya disease.

Direct versus indirect revascularization in the treatment of moyamoya disease

OBJECTIVEFor patients with moyamoya disease (MMD), surgical intervention is usually required because of progressive occlusion of the internal carotid artery. The indirect bypass method has been widely accepted as the treatment of choice in pediatric patients. However, in adult patients with MMD, the most effective treatment method remains a matter of debate. Here, the authors compared the clinical outcomes from MMD patients treated with either extracranial-intracranial arterial bypass (EIAB; 43 hemispheres) or modified encephaloduroarteriosynangiosis (mEDAS; 75 hemispheres) to investigate whether mEDAS is an effective surgical method for treating adults with symptomatic MMD.METHODSA comparative analysis was performed in patients treated using either mEDAS or EIAB. Collateral grading, collateral vein counting, and symptom analysis were used to assess the outcome of surgery.RESULTSSeventy-seven percent (58/75) of mEDAS cases and 83.7% (36/43) of EIAB cases in the analysis experienced improvement in their symptoms after surgery. Furthermore, patients in 98.7% (74/75) of mEDAS cases and those in 95.3% (41/43) of EIAB cases exhibited improved collateral grades. Increases in regions of perfusion were seen after both procedures.CONCLUSIONSModified EDAS and EIAB both result in positive outcomes for symptomatic adults with MMD. However, when considering the benefit of both surgeries, the authors propose mEDAS, a simpler and less strenuous surgery with a lower risk of complications, as a sufficient and safe treatment option for symptomatic adults with MMD.

Moyamoya disease: a case report

Moyamoya disease is a chronic, progressive occlusion of the circle of Willis arteries that leads to the development of characteristic collateral vessels seen on cerebral angiography. The appearance of these small, multiple vessels at the base of the brain resembles “puff of smoke”. The disease may develop in children and adults, but the clinical features differ. Children are likely to suffer with ischemic or haemorrhagic stroke. In the present report, authors describe a 7-year-old female with history of recurrent stroke apraxia and atrophy of left side with facial nerve palsy and was later on diagnosed with Moyamoya disease at HIMS Dehradun. Conclusion: Child with Moyamoya disease presents with recurrent episodes of sudden hemiplegia that might occur on alternate sides. The disease is progressive and dynamic in children. MRA clinches the diagnosis and neurosurgical intervention may be required as a part of definitive management.